PHEOCHROMACYTOMA

Signs and symptoms of pheochromacytoma usually follow a pattern of high blood pressure followed by low blood pressure. High blood pressure usually begins withn a change in breathingand a pounding or forceful heart. This may occur several times a week and last for 15 to 60 minutes. Often these episodes of high blood pressure are started by activities that press on the tumor, such as changes in position, exercise, lifting, defecation, emotional distress or anxiety.

What is Pheochromocytoma?

Pheochromocytoma is a tumor of the adrenal gland that causes excess release of the two hormones epinephrine and norepinephrine, which regulate heart rate and blood pressure.

The tumor may be single or multiple and usually develops in the medulla (center or core) of one or both adrenal glands. Sometimes the tumors occurs outside the adrenal gland, usually within the abdomen. Less than 10% of the tumors are malignant (cancerous).

The tumors may occur at any age, but they are most common in young to mid-adult life. A common clinical feature is a paroxysm (attack of symptoms) that may be frequent but sporadic, and may increase in frequency, duration, and severity.

What are the Common Symptoms of Pheochromocytoma?

  • Severe headache
  • Palpitations
  • Rapid heart rate
  • Sweating
  • Flushing
  • Chest pain
  • Abdominal pain
  • Nervousness
  • Irritability
  • Increased appetite
  • Loss of weight

How is Pheochromocytoma Diagnosed?

Vital signs (temperature, pulse, rate of breathing, blood pressure) reveal high blood pressure (hypertension) that may be sustained or episodic, rapid heart rate, and elevated temperature.

Tests include:

  • An adrenal biopsy that shows pheochromocytoma
  • A MIBG scintiscan that shows tumor
  • An MRI of abdomen that shows adrenal mass
  • An abdominal CT scan that shows mass
  • Elevated urine metanephrine
  • Elevated urine catecholamines
  • Abnormal results of a glucose test
  • Abnormal levels of catecholamines - blood

How is Pheochromocytoma Treated?

The definitive treatment is removal of the tumor by surgery. Continuous monitoring of all vital signs is necessary in the postoperative period in an intensive care unit. Stabilization of the person’s vital signs with medication prior to surgery is important and may require hospitalization. In the case of an inoperable tumor, management with medication is necessary. Radiation therapy or chemotherapy have not been effective in destroying the tumor.

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